Publications & Research

Nutrition in Clinical Practice : Official Publication of the American Society for Parenteral and Enteral Nutrition

Excerpt

Childhood and adolescence are important periods for bone development. Any disease that affects bone health has the potential to affect the bones not only in the short term but also later in life. Bone health abnormalities in patients with inflammatory bowel disease are being increasingly recognized. Screening the at-risk patient is important so that appropriate treatments can be instituted. Treatment options are limited to vitamin D and calcium supplementation, control of underlying disease activity, and appropriate physical activity. The role of bisphosphonates in these patients needs to be better studied, and treatment with bisphosphonates may be considered for some patients in consultation with a bone health expert.

Pediatric Crohn's disease (CD) is associated with growth, lean mass (LM), and fat mass (FM) deficits. This study assessed and identified determinants of changes in height and body composition in children with CD following.

Title

Effects of Sex, Race, and Puberty on Cortical Bone and the Functional Muscle Bone Unit in Children, Adolescents, and Young Adults.

Date

April 2010

Journal

The Journal of Clinical Endocrinology and Metabolism

Excerpt

Sex and race differences in bone development are associated with differences in growth, maturation, and body composition.

Title

Longitudinal Assessment of Bone Density and Structure in an Incident Cohort of Children with Crohn's Disease.

Date

February 2009

Journal

Gastroenterology

Excerpt

The impact of childhood Crohn's disease (CD) on volumetric bone mineral density (vBMD), bone structure, and muscle mass have not been established. The objective of this longitudinal study was to assess musculoskeletal outcomes in an incident cohort of children with CD using peripheral quantitative computed tomography (pQCT).

Title

Improvement in Biomarkers of Bone Formation During Infliximab Therapy in Pediatric Crohn's Disease: Results of the Reach Study.

Date

February 2009

Journal

Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association

Interpretation of Biomarkers of Bone Metabolism in Children: Impact of Growth Velocity and Body Size in Healthy Children and Chronic Disease.

Date

November 2008

Journal

The Journal of Pediatrics

Excerpt

OBJECTIVES: To determine the effects of growth, maturation, and whole body bone mineral content (WB-BMC) accrual on biomarkers of bone formation (bone-specific alkaline phosphatase [BSAP]) and resorption (urine deoxypyridinoline/creatinine [DPD]) in healthy children and children with Crohn's disease. STUDY DESIGN: BSAP and DPD were measured at baseline, with growth and dual energy x-ray absorptiometry (DXA) WB-BMC measured at baseline and 6 months in 202 control subjects and 110 subjects with Crohn's disease, ages 5 to 21 years. Multivariable linear regression identified determinants of biomarkers in control subjects and subjects with Crohn's disease. RESULTS: In control subjects, BSAP and DPD were significantly and independently associated with sex, Tanner stage, WB-BMC, height velocity, and WB-BMC accrual rates; these covariates explained 77% to 80% of the variability in the bone biomarkers, respectively. Subjects with Crohn's disease had lower height-for-age (P < .001) and WB-BMC-for-height (P <.05) than control subjects. Crohn's disease was associated with lower BSAP (P < .001) and greater DPD (P < .001), independent of growth, maturation, baseline WB-BMC, and WB-BMC accrual, compared with control subjects. CONCLUSIONS: These data illustrate the potential confounding effects of growth and WB-BMC on bone metabolism biomarkers in children. After adjustment for these effects, Crohn's disease was associated with lower biomarkers of bone formation and greater bone resorption.

Title

Gender Differences in Body Composition Deficits at Diagnosis in Children and Adolescents with Crohn's Disease.

Date

December 2007

Journal

Inflammatory Bowel Diseases

Excerpt

BACKGROUND: Childhood Crohn's disease (CD) is associated with poor growth and decreased body mass index (BMI); however, lean mass (LM) and fat mass (FM) deficits prior to therapy have not been characterized.Objectives: To quantify LM and FM in incident pediatric CD subjects and controls, and to identify determinants of LM and FM deficits. METHODS: Whole body LM and FM were assessed using DXA in 78 CD subjects and 669 healthy controls, ages 5-21 yr. Gender specific z-scores for LM (LM-Ht) and FM (FM-Ht) relative to height were derived using log linear regression models in the controls. Multivariate linear regression models adjusted for potential confounders. RESULTS: CD was associated with significantly lower height and BMI for age. Within CD subjects, FM-Ht and LM-Ht were significantly lower in females compared with males (FM-Ht z: -0.66+/-0.83 vs. -0.08+/-0.95, p<0.01; LM-Ht z: -1.12+/-1.12 vs. -0.57+/-0.99, p<0.05). In females, CD was associated with significantly lower LM-Ht (p<0.001) and FM-Ht (p=0.001), adjusted for age, race and Tanner stage, compared with controls. LM and FM deficits were significantly greater in older females with CD; 47% of adolescent females had LM-Ht<or=5th percentile. In non-black males, CD was also associated with lower LM-Ht (p<0.02); FM-Ht deficits were not significant. CONCLUSIONS: Incident CD was associated with significant LM deficits in males and females, and FM deficits in females. Future studies are needed to identify etiologies for the age and gender differences and to evaluate therapies for these deficits.

Title

Successful Revision of Portoenterostomy in an Infant with Biliary Atresia.

Date

September 2006

Journal

Journal of Pediatric Surgery

Excerpt

We present a case report of a boy with biliary atresia who, after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools. Nuclear medicine imaging study showed no excretion. Broad-spectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.

Title

Hepatosplenic T-cell Lymphoma in an Adolescent Patient After Immunomodulator and Biologic Therapy for Crohn Disease.

Date

June 2005

Journal

Journal of Pediatric Gastroenterology and Nutrition

Title

Impact of Intrapartum Antibiotics on the Care and Evaluation of the Neonate.

Date

December 2003

Journal

The Pediatric Infectious Disease Journal

Excerpt

BACKGROUND: Management of infants whose mothers receive intrapartum antibiotic prophylaxis (IAP) is controversial. In 1996 consensus guidelines for prevention of neonatal Group B streptococcal disease included an algorithm for management of infants whose mothers received IAP. To assess practices for testing and treatment of infants, we evaluated a population-based sample of deliveries to see whether excessive evaluation and treatment occurs after IAP. METHODS: Medical records for 869 deliveries in Connecticut during 1996 were sampled. IAP was administered in 96 full term deliveries. We excluded infants <37 weeks and those with intrapartum fever. We reviewed hospital records for infants born after IAP (n = 81) and a random sample of those not exposed (n = 180). Analyses were conducted with sample weights to account for unequal probability of selection. RESULTS: Infants whose mothers received IAP were more likely to have complete blood counts, (26% vs. 9% P = 0.05) but were no more likely to receive antibiotics in the first week of life (P = 0.48), have an intravenous catheter placed (P = 0.83), or to have other invasive procedures. Mean length of hospital stay was 6 h longer for infants born by vaginal delivery to mothers who had IAP (47.0 h) than for those without IAP (41.3 h) (P = 0.06). CONCLUSION: Despite concerns that IAP guidelines would result in excessive neonatal evaluations, infants sampled whose mothers received IAP were not more likely to undergo invasive procedures or to receive antibiotics. Consistent with the guidelines, collection of complete blood counts was more common among such infants.

Title

Cardiac Ischemia During Hemolytic Uremic Syndrome.

Date

December 2003

Journal

Pediatric Nephrology (berlin, Germany)

Excerpt

Increased thrombin generation and impaired fibrinolysis during Escherichia coli O157:H7-associated hemolytic uremic syndrome (HUS) plausibly diminish myocardial blood flow, but the frequency of cardiac ischemia during HUS is unknown. We identified a 9-year-old boy with HUS in whom myocardial diastolic dysfunction was demonstrated by echocardiography, who also had elevated serum troponin-I and creatine kinase MB mass. However, eight additional patients with HUS did not have elevated markers of cardiac injury. When present, elevated troponin-I should be considered to represent myocardial injury, and not attributed simply to renal insufficiency. It is possible that myocardial ischemia and secondary arrhythmias account for some sudden deaths that occur during acute HUS.

Cutaneous T-cell lymphoma is typically a clonal neoplasm of epidermotropic CD4+ T-lymphocytes that includes the entity mycosis fungoides (MF). After identification of patients with recurrent MF treated with total skin electron beam therapy (TSEBT) at the Yale University School of Medicine, this study attempted to compare T-cell receptor (TCR) gamma gene rearrangements via polymerase chain reaction (PCR) in both original and recurrent skin biopsies from these patients. Between 1974 and 1996, a total of 95 T2 MF patients were treated with TSEB, and four of these were identified for the study. Slides and tissue samples of both primary and recurrent skin biopsies for each patient were confirmed as being consistent with ME DNA for PCR was isolated from paraffin-embedded tissue samples. Using consensus primers that hybridize with conserved regions of the TCR gene, these regions of the genome were amplified. The PCR products were then analyzed by acrylamide gel electrophoresis. Of the primary and recurrent samples from four patients with a median disease-free interval (DFI) of 1222 days, only two showed evidence of a dominant TCR clone. A number of factors, including lack of sequence homology between the primers and the gene segments, the existence of multiple neoplastic cell lines, DNA degradation in the archival samples, and the presence of reactive as well as malignant lymphocytes, may have prevented the detection of dominant TCR rearranged clones in the samples. Despite the results of this study, TCR analysis via PCR and gel electrophoresis continues to be of utility in the evaluation of patients with MF when used in conjunction with other diagnostic modalities and in cases with nonspecific clinical, histopathological, and immunophenotyping findings.

Title

Cdc Group Iv C-2 Bacteremia in a Child with Recurrent Acute Monoblastic Leukemia.

Date

June 1999

Journal

The Pediatric Infectious Disease Journal

Title

Association of Chronic Kidney Disease with Muscle Deficits in Children.

Date

Journal

Journal of the American Society of Nephrology : Jasn

Excerpt

The effect of chronic kidney disease (CKD) on muscle mass in children, independent of poor growth and delayed maturation, is not well understood. We sought to characterize whole body and regional lean mass (LM) and fat mass (FM) in children and adolescents with CKD and to identify correlates of LM deficits in CKD. We estimated LM and FM from dual energy x-ray absorptiometry scans in 143 children with CKD and 958 controls at two pediatric centers. We expressed whole body, trunk, and leg values of LM and FM as Z-scores relative to height, sitting height, and leg length, respectively, using the controls as the reference. We used multivariable regression models to compare Z-scores in CKD and controls, adjusted for age and maturation, and to identify correlates of LM Z-scores in CKD. Greater CKD severity associated with greater leg LM deficits. Compared with controls, leg LM Z-scores were similar in CKD stages 2 to 3 (difference: 0.02 [95% CI: -0.20, 0.24]; P = 0.8), but were lower in CKD stages 4 to 5 (-0.41 [-0.66, -0.15]; P = 0.002) and dialysis (-1.03 [-1.33, -0.74]; P < 0.0001). Among CKD participants, growth hormone therapy associated with greater leg LM Z-score (0.58 [0.03, 1.13]; P = 0.04), adjusted for CKD severity. Serum albumin, bicarbonate, and markers of inflammation did not associate with LM Z-scores. CKD associated with greater trunk LM and FM, variable whole body LM, and normal leg FM, compared with controls. In conclusion, advanced CKD associates with significant deficits in leg lean mass, indicating skeletal muscle wasting. These data call for prospective studies of interventions to improve muscle mass among children with CKD.

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